Gene/Proteome Database (LMPD)
LMPD ID
LMP004694
Gene ID
Species
Homo sapiens (Human)
Gene Name
prenyl (decaprenyl) diphosphate synthase, subunit 2
Gene Symbol
Synonyms
C6orf210; COQ10D3; DLP1; bA59I9.3; hDLP1
Alternate Names
decaprenyl-diphosphate synthase subunit 2; decaprenyl pyrophosphate synthase subunit 2; subunit 2 of decaprenyl diphosphate synthase; decaprenyl pyrophosphate synthetase subunit 2; all-trans-decaprenyl-diphosphate synthase subunit 2
Chromosome
6
Map Location
6q21
EC Number
2.5.1.91
Summary
The protein encoded by this gene is an enzyme that synthesizes the prenyl side-chain of coenzyme Q, or ubiquinone, one of the key elements in the respiratory chain. The gene product catalyzes the formation of all trans-polyprenyl pyrophosphates from isopentyl diphosphate in the assembly of polyisoprenoid side chains, the first step in coenzyme Q biosynthesis. Defects in this gene are a cause of coenzyme Q10 deficiency.[provided by RefSeq, Oct 2009]
Orthologs
Proteins
| decaprenyl-diphosphate synthase subunit 2 | |
|---|---|
| Refseq ID | NP_065114 |
| Protein GI | 169808399 |
| UniProt ID | Q86YH6 |
| mRNA ID | NM_020381 |
| Length | 399 |
| RefSeq Status | REVIEWED |
| MNFRQLLLHLPRYLGASGSPRRLWWSPSLDTISSVGSWRGRSSKSPAHWNQVVSEAEKIVGYPTSFMSLRCLLSDELSNIAMQVRKLVGTQHPLLTTARGLVHDSWNSLQLRGLVVLLISKAAGPSSVNTSCQNYDMVSGIYSCQRSLAEITELIHIALLVHRGIVNLNELQSSDGPLKDMQFGNKIAILSGDFLLANACNGLALLQNTKVVELLASALMDLVQGVYHENSTSKESYITDDIGISTWKEQTFLSHGALLAKSCQAAMELAKHDAEVQNMAFQYGKHMAMSHKINSDVQPFIKEKTSDSMTFNLNSAPVVLHQEFLGRDLWIKQIGEAQEKGRLDYAKLRERIKAGKGVTSAIDLCRYHGNKALEALESFPPSEARSALENIVFAVTRFS | |
Gene Information
Entrez Gene ID
Gene Name
prenyl (decaprenyl) diphosphate synthase, subunit 2
Gene Symbol
Species
Homo sapiens
Gene Ontology (GO Annotations)
| GO ID | Source | Type | Description |
|---|---|---|---|
| GO:0005737 | IDA:HPA | C | cytoplasm |
| GO:0005759 | TAS:Reactome | C | mitochondrial matrix |
| GO:0046982 | IDA:HGNC | F | protein heterodimerization activity |
| GO:0000010 | IEA:Ensembl | F | trans-hexaprenyltranstransferase activity |
| GO:0050347 | IEA:Ensembl | F | trans-octaprenyltranstransferase activity |
| GO:0008299 | IDA:HGNC | P | isoprenoid biosynthetic process |
| GO:0051290 | IEA:Ensembl | P | protein heterotetramerization |
| GO:0050878 | IEA:Ensembl | P | regulation of body fluid levels |
| GO:0044281 | TAS:Reactome | P | small molecule metabolic process |
| GO:0006744 | IDA:HGNC | P | ubiquinone biosynthetic process |
KEGG Pathway Links
| KEGG Pathway ID | Description |
|---|---|
| hsa00900 | Terpenoid backbone biosynthesis |
| ko00900 | Terpenoid backbone biosynthesis |
BIOCYC Pathway Links
| BIOCYC Pathway ID | Description |
|---|---|
| PWY-5806 | all-trans-decaprenyl diphosphate biosynthesis |
REACTOME Pathway Links
| REACTOME Pathway ID | Description |
|---|---|
| REACT_160111 | Ubiquinol biosynthesis |
Domain Information
UniProt Annotations
Entry Information
Gene Name
prenyl (decaprenyl) diphosphate synthase, subunit 2
Protein Entry
DLP1_HUMAN
UniProt ID
Species
Human
Comments
| Comment Type | Description |
|---|---|
| Alternative Products | Event=Alternative splicing; Named isoforms=2; Name=1; IsoId=Q86YH6-1; Sequence=Displayed; Name=2; IsoId=Q86YH6-2; Sequence=VSP_017098, VSP_017099; |
| Catalytic Activity | (2E,6E)-farnesyl diphosphate + 7 isopentenyl diphosphate = 7 diphosphate + all-trans-decaprenyl diphosphate. |
| Disease | Coenzyme Q10 deficiency, primary, 3 (COQ10D3) [MIM |
| Function | Supplies decaprenyl diphosphate, the precursor for the side chain of the isoprenoid quinones ubiquinone-10. |
| Pathway | Cofactor biosynthesis; ubiquinone biosynthesis. |
| Sequence Caution | Sequence=AAH29491.1; Type=Frameshift; Positions=28; Evidence= ; Sequence=CAH73572.1; Type=Erroneous gene model prediction; Evidence= ; Sequence=CAI22355.1; Type=Erroneous gene model prediction; Evidence= ; |
| Similarity | Belongs to the FPP/GGPP synthase family. |
| Subcellular Location | Mitochondrion . |
| Subunit | Heterotetramer of 2 DPS1/TPRT and 2 DLP1 subunits. |
Identical and Related Proteins
Unique RefSeq proteins for LMP004694 (as displayed in Record Overview)
| Protein GI | Database | Accession | Length | Protein Name |
|---|---|---|---|---|
| 169808399 | RefSeq | NP_065114 | 399 | decaprenyl-diphosphate synthase subunit 2 |
Identical Sequences to LMP004694 proteins
| Reference | Database | Accession | Length | Protein Name |
|---|---|---|---|---|
| GI:169808399 | DBBJ | BAE48217.1 | 399 | subunit 2 of decaprenyl diphosphate synthase [Homo sapiens] |
| GI:169808399 | GenBank | EAW48400.1 | 399 | prenyl (decaprenyl) diphosphate synthase, subunit 2 [Homo sapiens] |
| GI:169808399 | GenBank | ACH16308.1 | 399 | Sequence 4 from patent US 7402413 |
| GI:169808399 | GenBank | AFL00053.1 | 399 | Sequence 4 from patent US 8163525 |
| GI:169808399 | SwissProt | Q86YH6.2 | 399 | RecName: Full=Decaprenyl-diphosphate synthase subunit 2; AltName: Full=All-trans-decaprenyl-diphosphate synthase subunit 2; AltName: Full=Candidate tumor suppressor protein; AltName: Full=Decaprenyl pyrophosphate synthase subunit 2 [Homo sapiens] |
Related Sequences to LMP004694 proteins
| Reference | Database | Accession | Length | Protein Name |
|---|---|---|---|---|
| GI:169808399 | DBBJ | BAG38145.1 | 399 | unnamed protein product [Homo sapiens] |
| GI:169808399 | GenBank | AAF97788.1 | 399 | candidate tumor suppressor protein [Homo sapiens] |
| GI:169808399 | GenBank | AAH39906.1 | 399 | Prenyl (decaprenyl) diphosphate synthase, subunit 2 [Homo sapiens] |
| GI:169808399 | GenBank | JAA01392.1 | 399 | prenyl (decaprenyl) diphosphate synthase, subunit 2 [Pan troglodytes] |
| GI:169808399 | GenBank | JAA21573.1 | 399 | prenyl (decaprenyl) diphosphate synthase, subunit 2 [Pan troglodytes] |
| GI:169808399 | GenBank | JAA21574.1 | 399 | prenyl (decaprenyl) diphosphate synthase, subunit 2 [Pan troglodytes] |